Patarata Eunice, Bernardino Vera, Martins Ana, Pereira Rui, Loureiro Conceição, Moraes-Fontes Maria Francisca
Unidade de Doenças Auto-imunes/Serviço Medicina 7.2, Hospital Curry Cabral, Centro Hospitalar Lisboa Central, Lisbon, Portugal.
Unidade de Cuidados Intensivos, Hospital Curry Cabral, Centro Hospitalar Lisboa Central, Lisbon, Portugal.
Case Rep Neurol. 2016 Dec 13;8(3):251-257. doi: 10.1159/000453545. eCollection 2016 Sep-Dec.
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rare condition characterized by emotional and behavioral disturbances, dyskinesias, and extrapyramidal signs. It occurs in young women of reproductive age and is classically described as a paraneoplastic phenomenon. We present a 36-year-old, HIV-positive female who was admitted to the hospital in an acute confusional state, with a stiff posture, periods of motor agitation, and myoclonic jerks of the hands. Her mental state progressively deteriorated. Without evidence of infection, the presence of anti-NMDAR antibodies both in serum and cerebrospinal fluid clinched the diagnosis of autoimmune encephalitis. No evidence of neoplastic disease was found, and the beneficial response to immunosuppressive therapy was exceptional. This is the first report of anti-NMDAR encephalitis in an HIV-infected individual, reminding us that autoimmune encephalitis should be included in the differential diagnosis of a young patient presenting in an acute confusional state.
抗N-甲基-D-天冬氨酸受体(抗NMDAR)脑炎是一种罕见疾病,其特征为情绪和行为障碍、运动障碍及锥体外系体征。该病发生于育龄期年轻女性,典型表现为副肿瘤现象。我们报告一例36岁HIV阳性女性,因急性意识模糊状态入院,伴有强直姿势、运动性激越发作及手部肌阵挛性抽搐。其精神状态逐渐恶化。在无感染证据的情况下,血清和脑脊液中均存在抗NMDAR抗体确诊为自身免疫性脑炎。未发现肿瘤性疾病证据,且对免疫抑制治疗的良好反应罕见。这是HIV感染个体中抗NMDAR脑炎的首例报告,提醒我们自身免疫性脑炎应纳入急性意识模糊状态年轻患者的鉴别诊断。